Paul K. Woolf

Symptomatic cardiac dysfunction in children with human immunodeficiency virus infection

Eight children with human immunodeficiency virus (HIV) infection had symptomatic cardiac dysfunction. The median age was 1.4 years (range 0.2 to 7.9 years). All had hepatosplenomegaly, fever, pneumonia with tachypnea, and tachycardia ascribed to infection and anemia. An S3 gallop was present in six of eight. All had normal creatine phosphokinase values. Chest x-rays did not aid in the diagnosis of cardiac dysfunction. ECG showed flattened T waves in five of eight with left ventricular hypertrophy, right ventricular hypertrophy, or both in seven of eight. Results of echocardiography showed decreased left ventricular function in all eight, despite anemia, with dilated left ventricular myopathy in six, concentric left ventricular wall thickening in two of eight, an enlarged right ventricle in two, and pericardial fluid in three. Medical therapy improved cardiac function in all. All patients subsequently died of noncardiac causes. Results of autopsies on four of eight patients showed focal myocarditis in two (with cytomegalovirus inclusions in one) and dilated cardiomyopathy in two others. We conclude: (1) Preexistent hepatosplenomegaly, fever, infection, and anemia result in physical findings that mimic findings of heart failure, thereby masking the occurrence of cardiac dysfunction; (2) an S3 gallop may indicate the presence of impaired heart function when other clinical signs are masked; (3) confirmation of cardiac compromise may be accomplished by noninvasive evaluation with echocardiography and (4) medical therapy can improve cardiac dysfunction in HIV-infected children.

Electrocardiographic findings in children with Lyme disease.

The incidence of cardiac involvement in Lyme disease (LD) has been estimated to be 4 to 10% in adults, with conduction and rhythm disturbances noted most frequently. To assess the frequency of electrocardiographic abnormalities in children with LD, we prospectively performed 12-lead electrocardiograms in 32 randomly selected children presenting with LD between May and September 1989. No patient had symptoms of cardiac involvement. Using defined diagnostic criteria, combining symptoms, signs, serology, and residence in or travel to an endemic area, 14 patients were classified as having definite LD and 10 were categorized as probable. The incidence of electrocardiographic abnormalities in the definite group was 29% (4/14), including two patients with 1“ atrioventricular block, one with left axis deviation, and one with ventricular ectopy. Thirty percent (3/10) of the probable group had abnormal ECGs, including one with ST-T wave abnormalities, one with prominent sinus arrhythmia, sinus bradycardia, and wandering atrial pacemaker, and one with ectopic atrial bradycardia. No patient required cardiac therapy.The incidence of abnormal ECG findings in this group of children with either probable or definite LD was thus 29%, with 1“ atrioventricular block noted most frequently. When the diagnosis of LD is highly suspected, an electrocardiogram may be a useful screening test for cardiac involvement.

Noncardiac chest pain in adolescents and children with mitral valve prolapse

Chest pain in adolescents and children is usually not of cardiac origin. Of cardiac conditions commonly linked to chest pain in childhood, mitral valve prolapse (MVP) is the most prevalent, but this association has recently been questioned. In light of recent reports of gastroesophageal sources of chest pain in adults with MVP, we performed a comprehensive gastroesophageal evaluation of 17 preadolescents and adolescents with mitral valve prolapse who had chest pain as their presenting symptom. Evaluation consisted of esophageal manometry, Bernstein test, esophageal pH probe, and/or esophagogastroscopy. Fourteen of the 17 patients had at least one abnormal finding. Five patients had esophagitis, five had gastritis, one had high-amplitude esophageal contractions, one had abnormal esophageal manometry with positive Bernstein test, one had esophageal reflux and positive Bernstein test, and one had abnormal manometry with esophageal reflux. The 13 patients with esophagitis, gastritis, reflux, or positive Bernstein test were treated with antacid, with resolution of chest pain in 12 patients. Two of these patients underwent follow-up endoscopy with documentation of improvement. The patient with high-amplitude esophageal contractions was treated with dicyclomine, which resulted in resolution of chest pain. The observation that the chest pain was not related to mitral valve prolapse is important in clinical practice and raises further questions as to whether mitral valve prolapse causes chest pain.

Life-threatening Dysrhythmias in Varicella Myocarditis

Varicella myocarditis is an unusual complication of a common childhood disease. Two patients with life-threatening dysrhythmias and circulatory failure are reported. One patient required permanent pacemaker implantation for acquired complete heart block, not previously described with varicella infection. Previously reported cases of varicella myocarditis are reviewed.

Variable arginine vasopressin levels in neonatal congestive heart failure

Arginine vasopressin levels in 17 neonates with cardiac disease were compared with control levels in 10 healthy newborn infants. Infants with congestive heart failure who were free of left ventricular outflow tract obstruction had a mean level of 80 +/- 18 pg/ml, which was significantly greater than the mean control level (p less than 0.001). Infants with congestive heart failure and left ventricular outflow tract obstruction had a mean vasopressin level of 3 +/- 0.7 pg/ml, which was lower than the mean control level of 6 +/- 0.7 pg/ml (p less than 0.05). The data suggest that impaired forward flow to high pressure sinoaortic and ventricular baroreceptors is necessary for vasopressin release in congestive heart failure. In left ventricular outflow tract obstruction with heart failure these receptors may be impaired or absent, leading to decreased vasopressin release. Low plasma arginine vasopressin may adversely affect circulatory homeostasis.

Elevated arginine vasopressin and lowered atrial natriuretic factor associated with hypertension in coarctation of the Aorta

Impairment of humoral and neural regulation of blood pressure may contribute to preoperative and postoperative hypertension in coarctation of the aorta and may also affect the release of vasopressin and atrial natriuretic factor. Because vasopressin and atrial natriuretic factor have potent vasoactive effects, we measured plasma vasopressin and atrial natriuretic factor levels by radioimmunoassay before operation and for 5 days after operation in 11 patients aged 9 months to 12 years undergoing coarctation repair and in 12 control patients undergoing other cardiovascular operations. Six patients in the coarctation group required minimal antihypertensive therapy (group I) and five required prolonged intravenous antihypertensive therapy (group II). Before operation, vasopressin levels correlated with systolic blood pressure for all patients in the coarctation group (r = 0.83, p < 0.01) whereas atrial natriuretic factor levels did not. Before operation, atrial natriuretic factor levels were lower (28 +/- 5 vs 41 +/- 7 and 50 +/- 8 pg/ml, p < 0.05) and vasopressin levels were higher (28 +/- 6 vs 5.4 +/- 0.9 and 7 +/- 3 pg/ml, p < 0.05) in group II than in group I or control patients. Vasopressin levels were higher (p < 0.05) on the day of operation and on postoperative days 2 through 5 in group II than in group I and in control patients. Atrial natriuretic factor levels were lower during the day of operation in group II than in group I or in control patients (26 +/- 7 vs 51 +/- 16 and 50 +/- 7 pg/ml, p < 0.05) and remained lower than control values on postoperative days 1 and 3 through 5. Elevated vasopressin and lowered atrial natriuretic factor levels may contribute to preoperative and postoperative hypertension in coarctation.

Safety and Efficacy of Outpatient Transseptal Radiofrequency Ablation of Atrioventricular Accessory Pathways

A retrospective analysis of 60 consecutive patients who underwent outpatient transseptal radiofrequency ablation of left sided accessory pathways at Westchester County Medical Center/New York Medical College from September 1994 to December 1997 was performed. Patients were followed for a mean duration of 22 months. No complications either local or related to the transseptal method were observed. All patients had successful ablation of the accessory pathway. One patient had a recurrence of symptoms. This study suggests transseptal radiofrequency ablation of the left sided accessory pathways to be safe, feasible and an effective procedure when performed in an outpatient setting. These results were obtained at a high volume center with experience using the transseptal technique.

ESOPHAGEAL DYSMOTILITY: A CAUSE OF CHEST PAIN IN CHILDHOOD MITRAL VALVE PROLAPSE (MVP)

Since the cause of chest discomfort in patients with mitral valve prolapse (MVP) is controversial, we prospectively measured esophageal motility in 14 children, 10 as normal controls and 4 with MVP and chest discomfort. Ages ranged from 10-14 yrs. All had clinical and echocardiographic evidence of MVP and normal left atrial size. None had rhythm disorders. Esophageal manometry was performed with the station pull-through technique and a triple-lumen perfused polyethylene catheter system. 10 wet swallows (3ml water) given at 30 sec. intervals were used to examine the esophageal body. Bernstein acid perfusion tests were performed on all patients.3 of 4 with MVP and chest discomfort had manometric abnormalities: Diffuse esophageal spasm was present in 1, hypotensive lower esophageal sphincter (<7mm Hg ) in 2. Bernstein tests reproducing chest discomfort were positive in the 2 with hypotensive lower esophageal sphincters. Intensive antacid therapy relieved onest discomfort in 2 patients and dicyclomine was effective in the patient with diffuse esophageal spasm. Control patients had normal esophageal manometric studies with negative Bernstein tests.Conclusions: 1. In some children with MVP, chest discomfort attributed to MVP may be due to esophageal dysmotility. 2. Children with MVP and chest pain may benefit from esophageal manometry and the Bernstein acid perfusion test prior to the initiation of therapy.

144 SERIM ARGININE VASOPRESSIN RESPONSES IN CONGENITAL HEART DISEASE

In addition to its antidiuretic properties, arginine vasopressin (AVP) is a potent vasoactive homone. If unopposed by baroreceptor mediated reflexes, even low serun levels of AVP nay exert pressor effects. Since early life is associated with an enhanced ability to release AVP and with an increase in its cardiovascular effects, we evaluated whether congestive heart failure (CHF) is a stimulus for AVP release. AVP levels were measured by RIA using the modified Bentonite technique in 40 infants and children divided into 5 groups: Group I (n = 7) with CHF; Group II (n = 6) with cyanotic heart disease; Group III(n = 3) with persistent neonatal pulmonary hypertension; Group IV (n = 12) stressed infants with respiratory disease but no heart disease; and Group V (n = 12) healthy unstressed controls. Group IV had statistically higher AVP levels (13 pcg/ml ± 6) when compared to Group V normals (6 peg/ml ± 2)(p ≤ 0.05). Groups II & III were not significantly different from Group V (7 pcg/ml ± 5 and 6 peg/ml ± 2 respectively). CHF patients (Group I) had variable AVP levels with peak levels markedly elevated above other groups (102 peg/ml ± 135), (p<0.05) and further increased with worsening clinical status.Additional data suggest that hyperosrrotic angiographic contrast madia increase AVP levels by 2 to 10 times pre-dye levels in most instances (n = 12 of 15 patients, p < 0.02).We conclude that AVP levels are increased in children with CHF and that diagnostic interventions such as angiography may further elevate AVP. This may result in increased cardiac afterload further impairing already diminished cardiac function.